An Open-label Extension Study of Inhaled Treprostinil in Subjects with Fibrotic Lung Disease

The INCREASE study (RIN-PH-201) demonstrated that inhaled treprostinil resulted in significant improvements in % predicted forced vital capacity (FVC) in subjects with pulmonary hypertension (PH) due to IPF, and it also reduced acute exacerbations of underlying lung disease in the overall PH associated with interstitial lung disease (PH-ILD) population. These improvements, combined with preclinical evidence of antifibrotic activity of treprostinil, suggest that inhaled treprostinil may offer a treatment option for patients with fibrotic lung disease, including IPF and PPF. Primary objective is to evaluate the long-term safety and tolerability of inhaled Treprostinil in subjects with IPF or PPF. Secondary objective is to provide or continue to provide inhaled Treprostinil for eligible subjects who participated in RIN-PF-301, RIN-PF-303 or RIN-PF-305. Up to 1850 subjects will be enrolled in the study.

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